Periorbital Necrotizing Sweet Syndrome: A Case Report and Literature Review

Necrotizing periorbital Sweet syndrome is an uncommon mimic of necrotizing fasciitis that requires contrasting management. We describe a 60-year-old man with rapidly progressive unilateral eyelid edema, pain, and necrosis unresponsive to broad-spectrum antibiotics. Computed tomography showed periorbital soft-tissue swelling with gas. Two debridements for presumed necrotizing fasciitis yielded "dishwater" fluid; histopathology and special stains revealed a dense neutrophilic dermatosis without organisms, and cultures grew only commensals. He improved promptly after high-dose corticosteroids. Hematologic evaluation for escalating leukocytosis uncovered acute myeloid leukemia with t(6;9) and neuroblastoma RAS viral oncogene homolog mutation. A targeted literature synthesis showed frequent initial misdiagnoses as cellulitis or necrotizing fasciitis, unnecessary debridement, common association with myeloid neoplasia, and rapid steroid response. Periorbital sweet syndrome should be considered when cultures are sterile and clinical status worsens despite antibiotics; early biopsy and empiric corticosteroids can avert morbidity and unmask occult malignancy.